Oral Pathology Chapter 16 Flashcards
What is the name of the pathology that is a group of inherited conditions in which two or more ectodermal anatomic structures fail to develop?
Ectodermal dysplasia
What is the best known type of ectodermal dysplasia?
Hypohidrotic Ectodermal Dysplasia
What pathology is an autosomal dominant disease that results from defects in keratins and appears as thickened, symmetrical, white, corrugated, diffuse plaques on the bilateral buccal mucosa?
White Sponge Nevus
What is the scientific term that describes the idea of a genetically determined skin disorder?
Genodermatosis
What pathology is recognized by freckle-like lesions on the hands, perioral skin, and oral mucosa?
Peutz-Jeghers Syndrome
What side effect does Peutz-Jeghers Syndrome have?
Intestinal Polyposis
What is the significance for patients that develop Peutz-Jeghers Syndrome?
They have a predisposition to develop cancer
What type of growth are the intestinal polyps in Peutz-Jeghers Syndrome?
Hamartomatous
What digestional problem do patients with Peutz-Jeghers Syndrome often have?
Intussusception (proximal bowel becomes telescoped into distal portion)
How much more likely are Peutz-Jeghers syndrome patients to develop a malignancy later in life than an ordinary person?
18 X more likely
What other problem develops in the digestion tract for Peutz-Jeghers Syndrome patients?
GI adenocarcinomas develop (note that they do NOT develop from polyps)
What pathology is associated with CNS manifestions, mental retardation, seizures, potato-like growths, angiofibromas of the skin, ungual fibromas and ash-life/shagreen patches on the skin?
Tuberous Sclerosis
What rare side effects can be associated with Tuberous Sclerosis?
Cardiac Rhabdomyoma
What effect does Tuberous Sclerosis have on the kidneys?
Angiomyolipomas form there
What are the oral manifestations of Tuberous Sclerosis (2)?
- Developmental enamel pitting on facials of anterior permanent teeth and 2. Multiple Fibrous Papules
What are the odds of a patient with Tuberous Sclerosis developing tubers, seizures and mental retardation?
85%, 75% and 40%
What disease is related to autoantibodies against the desmosomes?
Pemphigus Vulgaris
What skin lesions are present in Pemphigus Vulgaris (2)?
- Flaccid vesicles and 2. bullae
What is the name of the sign that signifies the induction of bulla on normal-appearing skin?
Nikolsky sign
What is the term given for the “falling apart” of epithelium in Pemphigus Vulgaris?
Acantholysis
What is the name of free-floating cells found in Pemphigus Vulgaris and some other pathologies (Herpes, etc.)?
Tzanck Cells
What is the name of the familial disease that passes Pemphigus Vulgaris around in the same family?
Hailey-Hailey disease
What disease causes problems in hemidesmosomal binding and creates vesicles/bullae?
Mucous Membrane Pemphigoid
What intraoral lesion is almost pathognomonic for Mucous Membrane Pemphigoid?
Intraoral blood blister
What is the other term that is used for Mucous membrane pemphigoid?
Cicatricial Pemphigus
What is the most significant complication of Mucous membrane pemphigoid?
Ocular Involvement (25%)
Scarring in Mucous Membrane Pemphigoid causes what to occur in the eyelids?
Eyelids turn inward on themselves (Entropion)
What two proteins are found along the basement membrane in Mucous Membrane Pemphigoid?
C3 and IgG
What disease is a blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis?
Erythema Multiforme
What can be said of 50% of Erythema Multiforme cases?
Clinicians can identify a preceding infection or exposure to a new medication
What two infections have the greatest chance of developing into Erythema Multiforme?
- HSV and 2. Mycoplasma pneumoniae
What percentage of Erythema Multiforme patients have experienced a HSV infection previously?
50%
What three gradients of Erythema Multiforme have been classified?
- Erythema multiforme minor, 2. Erythema Multiforme major and 3. Toxic epidermal necrolysis
What is the more common name used for Erythema Multiforme Major?
Stevens-Johnson Syndrome
What is the other name for toxic epidermal necrolysis?
Lyell’s Disease
What are the demographics of those most likely to experience Erythema Multiforme?
20-30 with males more predisposed
What “warning” do patients have before they develop Erythema Multiforme?
Prodrome Symptoms about a week in advance
How long does Erythema Multiforme last?
2-6 weeks typically
What skin lesion is particularly unique to Erythema Multiforme?
Target Lesions
What two areas of the mouth cannot have Erythema Multiforme?
- Gingiva and 2. Hard Palate
What triggers Toxic Epidermal Necrolysis?
Always a drug
What is the mortality of Erythema Multiforme Major?
10%
What is the mortality of Toxic Epidermal Necrolysis?
35%
What pathology is a common, chronic dermatologic disease that can affect oral mucosa?
Lichen Planus
What is the other name that Lichen planus is known as?
Lichenoid Mucositis
What percentage of the population have lichen planus?
1%
What are the four P’s that describe lichen planus?
- Purple, 2. Pruritus, 3. Polygonal and 4. Papules
What is the lacelike network of white lines found in the skin papules of lichen planus?
Wickham’s Striae
What are the two forms of lichen planus?
- Reticular and 2. Erosive
What form of Lichen planus is seen on the buccal mucosa bilaterally and is seen as Wickham’s Striae?
Reticular
What form of lichen planus can be describe as presenting with atrophic lesions, erythematous areas with central ulceration and periphery that is usually bordered by fine, white, radiating striae?
Erosive
If erosive Lichen Planus is limited to the gingiva, what is it known as?
Desquamative Gingivitis
What may be indistinguishable from lichen planus on the gingiva (2)?
- Pemphigus and 2. Pemphigoid
What is the name of degenerating keratinocytes within the epithelium in lichen planus?
Civatte bodies
What are the two keys to not needing a biopsy for lichen planus?
- The lesions are bilateral and 2. The lesions are asymptomatic
What percentage of women with erosive lichen planus have vaginal lesions?
28%
What does the acronym STAY stand for with lichen planus?
Stress, Trauma, Advil (any NSAIDs) and Yeast
What pathology is a classic example of an immunologically mediated condition and is the most common collagen vascular/connective tissue disease in the US?
Lupus Erythematous
What two forms of Lupus Erythematous do we care about?
- Systemic lupus erythematous and 2. Chronic Cutaneous Lupus Erythematous
What is the name of the distinctive rash that forms in Lupus Erythematous?
Malar rash
What makes the rash worsen in Lupus Erythematous?
Sunlight
What percentage of Lupus Erythematous patients have kidney problems and develop Malar rashes?
50%
What is the most significant risk of Lupus Erythematous?
Risk of kidney damage
Up to 50% of Lupus Erythematous patients have vegetations affecting heart valves termed what?
Libman-Sacks endocarditis
How do skin lesions present in Chronic Cutaneous Lupus Erythematous?
Discoid lupus erythematous lesions
How do skin lesions start in Lupus Erythematous?
Scaly, erythematous patches
What two types of drugs should be used in mild Systemic Lupus Erythematous?
- NSAIDs and 2. antimalarial drugs
What antimalarial drug is typically used for treating Systemic Lupus Erythematous?
Hydroxychloroquine
What side effect does Hydroxychloroquine have?
Turns the palate blue
What two medication classes are used to treat more severe Systemic Lupus Erythematous?
- Corticosteroids and 2. Immunosuppressive agents
What medication class is used to treat patients with Chronic Cutaneous Lupus Erythematous?
Topical Corticosteroids
What is the pathology that presents as a disease where dense collagen is deposited in the tissues of the body in extraordinary amounts?
Systemic Sclerosis
What is the first sign of systemic sclerosis called?
Raynaud’s Phenomenon
What two things are known to trigger Raynaud’s Phenomenon?
- Emotional Distress or 2. Exposure to cold
What strange symptom manifests in the patient’s hands?
Resorption of terminal phalanges and flexion contraction to make shortened, claw-like fingers
What facial effect happens in systemic sclerosis?
Mask-like faces
What happens to the nose in systemic sclerosis?
Alae become atrophied resulting in a pinched appearance
What four organs are you concerned about if a patient has Systemic Sclerosis?
- Lung, 2. Heart, 3. Kidney and 4 GI tract
Should an organ become affected by systemic sclerosis, what will happen?
It will fail within 3 years
What happens to patients with systemic sclerosis regarding their mouths?
Microstomia from collagen deposition
What is the name of the form of Systemic Sclerosis that only affects an area and looks as though it were a scar from a sword?
Localized Scleroderma
What does CREST stand for in CREST syndrome?
C - Calcinosis cutis, R - Raynaud’s phenomenon, E - Esophageal dysfunction, S - Sclerodactyly and T - Telangiectasia
What is the name given to movable, nontender, subcutaneous nodular calcifications found in CREST syndrome?
Calcinosis Cutis
What is Reynaud’s Phenomenon?
When hands/feet turn white when cold, then blue, and then turn red when heated up and hurt
What term describes permanent flexure of fingers resulting in “claw” deformities?
Sclerodactyly
What term describes numerous scattered red papules, 1-2 mm in size, that blanch and are frequently found in the vermilion zone of the lips/facial skin?
Telangiectasias
What is a side effect of CREST syndrome that should be monitored for?
Pulmonary Hypertension
